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JOURNAL OF MEDICALCASE REPORTS
Alshammari et al. Journal of Medical Case Reports 2012,
6:410http://www.jmedicalcasereports.com/content/6/1/410
CASE REPORT Open Access
Clival chordoma with an atypical presentation: acase reportJaber
Alshammari1, Philippe Monnier1, Roy T Daniel2 and Kishore
Sandu1,3*
Abstract
Introduction: Clival chordomas present with headache, commonly
VI cranial nerve palsy or sometimes with lowercranial nerve
involvement. Very rarely, they present with cerebrospinal fluid
rhinorrhoea due to an underlyingchordoma-induced skull base
erosion.
Case presentation: A 60-year old Caucasian woman presented with
meningitis secondary to cerebrospinal fluidrhinorrhoea. At first,
radiological imaging did not reveal a tumoral condition, though
intraoperative exploration andtissue histology revealed a chordoma
which eroded her clivus and had a transdural extension.
Conclusion: Patients who present with meningitis and
cerebrospinal fluid rhinorrhoea could have an underlyingerosive
lesion which can sometimes be missed on initial radiological
examination. Surgical exploration allowscollecting suspicious
tissue for histological diagnosis which is important for the actual
treatment. A revisionendoscopic excision of a clival chordoma is
challenging and has been highlighted in this report.
IntroductionChordomas are rare, slow-growing malignant bony
tu-mors that arise from remnants of the notocord and ac-count for
approximately 1% of intracranial tumors [1].Approximately 35% to
40% of these tumors occur in theskull base, where they typically
involve the clivus [1-3].While these tumors rarely metastasize to
distant sites,they are locally aggressive and tend to recur after
surgi-cal resection, with a life expectancy of less than 10
yearsafter diagnosis [4]. Most commonly, the patient presentswith
headache, diplopia secondary to VI cranial nerveparesis and visual
changes including blurring or some-times loss of vision [5]. The
patient may present withmultiple lower cranial nerve palsy symptoms
such asfacial numbness and asymmetry, dysphagia, hoarsenessand
speech problems. Finally, large tumors may causebrainstem
compression and patients may present withlong tract signs and
ataxia [6,7]. Epistaxis as a rare pre-sentation has also been
reported [8].Cerebrospinal fluid (CSF) rhinorrhea has been very
rarely
described as a presenting symptom [9,10]. We present a
* Correspondence: [email protected] of
Otorhinolaryngology, University Hospital - CHUV,Lausanne,
Switzerland3Department of Otorhinolaryngology, Valais State
Hospital - CHCV,Sion, SwitzerlandFull list of author information is
available at the end of the article
© 2012 Alshammari et al.; licensee BioMed CeCreative Commons
Attribution License (http:/distribution, and reproduction in any
medium
case report of a patient who presented initially with
menin-gitis and later was diagnosed to have a clival chordoma.We
discuss the treatment modalities and present a syste-matic review
of the literature.
Case presentationA 60-year-old Caucasian woman, living alone and
pre-viously in good health, was found unconscious at homeand
fortunately helped by her friends and the emergencymedical team.
One week before this incident, she hadinfrequent episodes of watery
nasal discharge that in-creased with forced sneezing and cough. On
her arri-val to our clinic, she was confused and febrile. A
cranialnerve examination was normal. A computed tomography(CT) scan
(Figure 1) of her brain and paranasal sinusesshowed pneumocephalus
in the ventricles, hydrocephalusand an air-fluid level in the right
sphenoid sinus indicatinga CSF leak. There was a bony defect of the
clivus withsclerotic edges. A lumbar puncture and analysis of
herCSF confirmed bacterial meningitis and our patient
wasimmediately started on ceftazidime 2g three times a day.Magnetic
resonance imaging (MRI) (Figures 2 and 3)showed an osteolytic,
irregular lesion occupying theupper clivus and measuring
13×12×16mm. It was hype-rintense on T2-weighted images and
hypointense onT1-weighted images. The lesion showed no
enhancement
ntral Ltd. This is an Open Access article distributed under the
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Figure 3 T1-weighted sagittal images showing an osteolyticlesion
of the clivus with no contrast enhancement.
Figure 1 Computed tomography scan showing presence of airin the
subarachnoid spaces and ventricles.
Alshammari et al. Journal of Medical Case Reports 2012, 6:410
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with administration of gadolinium. An endonasal trans-sphenoidal
endoscopic exploration of the skull base defectand closure of the
CSF fistula was planned. A bony defectand CSF leak was visualized
on the postero-inferior wallof her right sphenoid sinus at the
posterior cortical marginof her clivus. Further exploration
revealed suspicious
Figure 2 T2-weighted sagittal images showing erosion of
theposterior wall of the clivus and hyperintensity suggestive
ofcerebral spinal fluid, with a fluid level in the sphenoid
sinus.Note also, the presence of pneumocephalus with air in
thesubarachnoid spaces and the ventricle.
tissue adjacent to the dural defect that was just anterior toher
basilar artery. This tissue was carefully dissected andsent for
histopathological examination. The skull base de-fect was repaired
using fascia lata and the sphenoid sinuswas obliterated with fat
and fibrin glue TISSEELW. Histo-pathologic analysis of the biopsied
tissue showed darkgiant oval nuclei, with vacuolated or granular
eosinophiliccytoplasm. The cells were arranged in epithelial
cords,and on immunohistochemistry were strongly positive forS-100
protein and epithelial membrane antigen, therebyconfirming
diagnosis of a chordoma in the clival region.Three months later,
our patient underwent an MRI
(Figures 4 and 5) to re-define the presence and extent ofthe
clival chordoma. In the intervening period she wasasymptomatic and
did not have any CSF leak. A transnasalendoscopic resection of the
chordoma aided by neuro-navigation was planned. Surgery began with
a completeright-side ethmoidectomy. Posterior septectomy allowed
a'two nostrils - four hands’ technique for tumor removal. Aradical
vomerectomy was performed. The entire anteriorwall of her sphenoid
sinus and her sphenoidal septumwere excised. During a revision
endoscopic exploration itis extremely important to identify the
bony edges circum-ferentially around the earlier defect and
gradually definethe bone and soft tissue junction, which
necessitated re-moval of the upper two-thirds of her clivus. The
nextstep was to identify the sella, optic nerve and the caro-tid
impressions bilaterally within her sphenoid sinus. Sub-sequently,
the normal dura and fibrocicatricial tissuesurrounding the chordoma
were identified. An en blocexcision of the chordoma was done,
anticipating herbasilar artery to be just posterior to the tumor.
Recons-truction of the defect was performed using fascia lata,
fat
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Figure 4 T2-weighted sagittal images showing a
predominantlyhyperintense lesion involving the posterior wall of
the clivus justanterior to the basilar artery. The sphenoid sinus
shows evidenceof packing done during the first surgery with
inflammatory changes.
Figure 6 T2-weighted sagittal magnetic resonance scan showstotal
excision of the lesion with the packing material seen inthe
posterior part of the clivus in the extradural plane.
Alshammari et al. Journal of Medical Case Reports 2012, 6:410
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and fibrin glue. A lumbar drain was inserted preopera-tively and
used to drain CSF in the postoperative period.It was removed on the
fifth postoperative day. Broad spec-trum antibiotics (ceftazidime
2g three times a day) were
Figure 5 T1-weighted gadolinium enhanced images show
ahypointense lesion of the clivus more towards the right sidewith
destruction of the bony margins of the clivus. The lesion isfound
just anterior to the basilar artery. There was no
contrastenhancement of the lesion.
given for 10 days. Our patient had an excellent recoverywith no
complications. The follow-up scans showed com-plete excision of the
tumor and no other adjuvant therapywas instituted. Our patient was
discharged on the 12th
postoperative day and a systematic clinico-radiological
fol-low-up is planned. She was totally asymptomatic at thelatest
follow-up (three months after surgery) with an MRI(Figures 6 and 7)
showing complete excision of the tumor.
Figure 7 T1-weighted fat suppressed images show no evidenceof
residual or recurrent tumor.
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Alshammari et al. Journal of Medical Case Reports 2012, 6:410
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DiscussionChordomas were first described by Virchow in 1857as
tumors made up of vacuolated or ‘physaliferous’ cellsderived from
rests of embryonic notocord along themidline central nervous system
axis [11]. Chordomas caninvade and metastasize to bony structures
like the sacro-coccyx, skull base and vertebrae [11]. The
histologicalappearance of a chordoma includes pleomorphic cellswith
dark nuclei and vacuolated or granular cytoplasm setwithin an
abundant myxoid matrix. Neoplastic cells arearranged in epithelial
cords separated by mucinous mate-rial, which is a classic feature
of chordomas. On immuno-histochemistry, the cells are positive for
S-100 protein andepithelial membrane antigen [8]. The symptoms of a
clivalchordoma depend mainly on the site of the tumor and
theadjacent structures. Headache, visual changes and cranialnerve
palsies are the most frequent symptoms [4], thoughrare
presentations like CSF rhinorrhea and epistaxis havealso been
reported [8,10].For diagnosis, it is mandatory to do a CT scan and
MRI
for all skull base tumors, although there are no
reliablediagnostic features that allow differentiation between
thesetumors [11]. Generally, MRI is better for defining theexact
position of the brainstem and the optic chiasmarelative to the
tumor with added information about tumorextension into the
nasopharynx and cavernous sinus. Italso demonstrates the position
of the cavernous internalcarotid, vertebral and basilar arteries in
relation to thetumor [12]. CT is better than MRI in demonstrating
tu-moral calcification and associated bone destruction.Clival
chordomas can be managed by a variety of conven-
tional surgical approaches: transcranial,
transsphenoidal,transoropharyngeal and maxillary osteotomy
approaches[13]. Transcranial approaches involve brain retraction
andhave increased risks of cerebral edema and hematoma,apart from
carotid, basilar artery and optic nerve trauma.These complications
can be greatly reduced with anterior(transnasal, transoral and
transfacial) approaches [13]. Cur-rently, endoscopic surgery has
opened a new avenue in themanagement of clival chordomas, not only
as a direct sur-gical access but also by providing an excellent
visualizationof the clivus and surrounding structures, especially
the an-terior dura and the basilar artery. The surgical
procedures,techniques and selection of the endoscopic surgical
stra-tegies for preservation of the vital anatomic structures
aredescribed in detail in the literature [13].This case report is
interesting on several counts. In ge-
neral, patients with clival chordomas present with head-ache or
cranial nerve palsies. Our patient was hospitalizedas an emergency
case with meningitis, which in turn wassecondary to a CSF leak
caused by skull base erosion.MRI showed an osteolytic, irregular
lesion occupyingher upper clivus that was hyperintense on
T2-weightedimages and hypointense on T1-weighted images,
showing
no enhancement with administration of gadolinium. Nei-ther CT
nor MRI images revealed any obvious tumormass. Transnasal
endoscopic exploration allowed us toclose the skull base defect and
collect local soft tissue forhistological analysis, which showed
evidence of a chor-doma. A second surgery for an en bloc resection
of thechordoma was performed three months after the
initialintervention. It is quite possible that, right at the first
pres-entation, our patient had a clival chordoma with skull
baseerosion, but it did not enhance on contrast administrationand
hence could not be diagnosed in the earlier CT andMRI scans. An
intradural extension of the tumor led to aCSF leak and subsequent
meningitis. A systematic explo-ration of her skull base allowed us
to send local soft tissuefor histological analysis, which confirmed
the diagnosis ofa chordoma and allowed us to plan the subsequent
radicalendoscopic excision.A revision endoscopic skull base surgery
can be diffi-
cult due to severe postoperative fibrocicatricial tissue asa
result of the packing material (fat, fascia lata) used forthe CSF
leak closure and skull base reconstruction du-ring the prior
surgery. During such revision explorations,it is of paramount
importance to define the tumoral softtissue and bone erosion
limits, followed by identificationof the important landmarks within
the sphenoid sinus.Neuronavigation helps in identifying vital
anatomicalstructures at the anterior skull base and avoids
intraopera-tive complications, thereby facilitating a complete
tumorremoval. The two nostrils - four hands technique facili-tates
better transnasal instrumentation allowing a com-plete tumor
excision and efficient reconstruction of theskull base defect.The
role of radiation therapy has been extremely con-
troversial in the treatment of clival chordomas. Con-ventional
radiation does not appear to have an effect onsurvival in the study
published by Colli and Al-Mefty[14]. Results of this study
indicated that the patient over-all survival significantly improved
with a radical resec-tion of the tumor combined with postoperative
protonbeam radiotherapy. There is no study in the
literaturecomparing the results of surgery alone and surgery
withpostoperative radiotherapy. Finally, with a limited numberof
cases in the literature, we cannot conclude on the mosteffective
and ideal management of these cases, thoughmost authors agree that
complete surgical excision of thetumor mass with an adequate margin
provides the bestchance for a recurrence-free survival [14,15].
ConclusionPatients with a clival chordoma commonly present
withheadaches, visual changes, cranial nerve palsies and, rare-ly,
with CSF rhinorrhea and epistaxis. Patients who pre-sent with
meningitis secondary to a CSF leak may havean underlying erosive
lesion that can be missed on initial
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Alshammari et al. Journal of Medical Case Reports 2012, 6:410
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radiological examination. A revision transnasal
endoscopicexcision of a clival chordoma is challenging and
warrantscollaborative efforts between the ear, nose and throat
sur-geons and neurosurgeons.
ConsentWritten informed consent was obtained from the patientfor
publication of this manuscript and accompanyingimages. A copy of
the written consent is available for re-view by the Editor-in-Chief
of this journal.
Competing interestsThe authors declare that they have no
competing interests.
Authors’ contributionsJA - collection of data, manuscript
writing. PM - analysis and editing.RTD - analysis and editing the
manuscript. KS - collection of data, manuscriptwriting, analysis
and editing the manuscript. KS was the major contributor inwriting
the manuscript. All authors read and approved the final
manuscript.
Author details1Department of Otorhinolaryngology, University
Hospital - CHUV,Lausanne, Switzerland. 2Department of Neurosurgery,
University Hospital- CHUV, Lausanne, Switzerland. 3Department of
Otorhinolaryngology,Valais State Hospital - CHCV,Sion,
Switzerland.
Received: 12 July 2012 Accepted: 16 October 2012Published: 29
November 2012
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doi:10.1186/1752-1947-6-410Cite this article as: Alshammari et
al.: Clival chordoma with an atypicalpresentation: a case report.
Journal of Medical Case Reports 2012 6:410.
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AbstractIntroductionCase presentationConclusion
IntroductionCase
presentationDiscussionConclusionConsentCompeting interestsAuthors’
contributionsAuthor detailsReferences